Vasomotor phenomenon due to a disorder of the autonomic nervous system, characterized by a red, asymmetrical skin coloration (without crossing the median line) at the level of the face, neck and upper part of the chest.

The cause is usually a dysfunction of the cervical sympathetic system. Paradoxically, it is the 'red' side which is healthy. The affected side appears pale.

There are:

-        primary or idiopathic cases: no identified cause

-        secondary cases (15 %): tumor compressing the nervous trunks of the neck, carotid dissection in its cervical section

-        iatrogenic cases:

-        flush to the left hemiface after the dissection of a right cervical mass

-        at the level of the whole right hemibody following resection of a left paracarotid cervical neuroblastoma

-        following thoracoscopy for the cure of an esophageal atresia

-        during a thoracic epidural or paravertebral block

-        following placement of a central venous catheter: hematoma, trauma ?

Anesthetic implications:

according to the initial cause

References :

-        Turco GR, Farber NE.
Postoperative autonomic deficit: a case of Harlequin syndrome. nesthesiology 1996; 85: 1197-9.

-        Pada GS, Cruz OA, Silen ML, Krock JL.
Skin conductance responses in paediatric Harlequin syndrome.
Paediatr Anaesth 1999 ; 9 : 159-62.

-         Mascianica KA, Smigiel R, Patkowski D.
The Harlequin phenomenon after thoracoscopic repair of oesophageal atresia and tracheoesophageal fistula: is there a coincidence ?
J Ped Surg Case Reports 2015; 3:473-5

-        Thomas JJ, Polaner D.
Harlequin syndrome associated with epidural analgesia.
Anesthesiology 2015; 123: 118.

-        Nagasaka Y, Wasner G, Sharma B, Fleischmann K.
Harlequin syndrome after thoracic paravertebral block.
A&A Case Reports 2016; 6: 48-51

-        Kydes AT, Kelley AM, Pandya SR, Barst SM.
Postoperative Harlequin syndrome: case report of a rare but clinically striking condition.
A&A Practice 2018; 10: 215-7

-        Rovner MS, Redding AT, Jacobs Wolf B, Wharton JA, Risely CJ, Furse CM.
Detection of subclinical Harlequin syndrome in pediatric patients.
Pediatr Anesth 2020 ; 30 : 592-8.

Updated: June 2020